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Lee RK, Gallagher JJ, Ejike JC, Hunt L. Intra-abdominal Hypertension and the Open Abdomen: Nursing Guidelines From the Abdominal Compartment Society. Crit Care Nurse 2020; 40:13-26. [PMID: 32006038 DOI: 10.4037/ccn2020772] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022]
Abstract
Intra-abdominal hypertension has been identified as an independent risk factor for death in critically ill patients. Known risk factors for intra-abdominal hypertension indicate that intra-abdominal pressures should be measured and monitored. The Abdominal Compartment Society has identified medical and surgical interventions to relieve intra-abdominal hypertension or to manage the open abdomen if abdominal compartment syndrome occurs. The purpose of this article is to describe assessments and interventions for managing intra-abdominal hypertension and open abdomen that are within the scope of practice for direct-care nurses. These guidelines provide direction to critical care nurses caring for these patients.
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Affiliation(s)
- Rosemary K Lee
- Rosemary K. Lee is an acute care nurse practitioner and clinical nurse specialist at Baptist Health South Florida, Coral Gables, Florida
| | - John J Gallagher
- John J. Gallagher is a clinical nurse specialist and trauma program coordinator, Penn Presbyterian Medical Center, Philadelphia, Pennsylvania
| | - Janeth Chiaka Ejike
- Janeth Chiaka Ejike is an associate professor of pediatrics, pediatric critical care medicine practitioner, and Program Director of the Pediatric Critical Care Medicine Fellowship at Loma Linda University Children's Hospital, Loma Linda, California
| | - Leanne Hunt
- Leanne Hunt is a senior lecturer at Western Sydney University and a registered nurse at Liverpool Hospital, Sydney, Australia
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Lode HN, Henze G, Siebert N, Ehlert K, Barthlen W. Management of tumor rupture and abdominal compartment syndrome in an infant with bilateral high risk stage 4 neuroblastoma: A case report. Medicine (Baltimore) 2019; 98:e16752. [PMID: 31441848 PMCID: PMC6716702 DOI: 10.1097/md.0000000000016752] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
RATIONALE Tumor rupture and bleeding at initial presentation of infants with neuroblastoma (NBL) is a rare, but life threatening condition and challenge in pediatric oncology. Here, we report successful multidisciplinary management of an abdominal compartment syndrome as a result of tumor rupture and bleeding in an infant with bilateral high risk stage 4 NBL. PATIENT CONCERNS The patient was admitted to a cooperating hospital with vomiting, failure to thrive and a large mass in the abdomen and was then referred to our center. DIAGNOSES Stage 4 NBL with MYC-N amplification and 1p36 deletion was diagnosed in an 11 months old girl. Due to rapid and massive tumor growth she developed abdominal compression with renal failure, severe bleeding, and tumor lysis syndrome (TLS). INTERVENTIONS Surgical decompression by enterostomy, local, and systemic bleeding control with platelets and coagulation factors, antiinfective and TLS therapy were effective in stabilizing the patient's condition. This allowed initiation of the multimodal antineoplastic treatment according to protocol NB 2004. OUTCOMES Mechanical ventilation was stopped after 11 days, the abdominal wall was closed 3 months after the start of therapy, and treatment according to the protocol be started and successfully completed. LESSONS Only the immediate, coordinated multidisciplinary intervention managed to overcome the life-threatening abdominal compartment syndrome and its associated problems, eventually enabling successful curative treatment.
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Affiliation(s)
- Holger N. Lode
- University Medicine Greifswald, Department of Pediatric Hematology and Oncology
| | - Günter Henze
- University Medicine Greifswald, Department of Pediatric Hematology and Oncology
| | - Nikolai Siebert
- University Medicine Greifswald, Department of Pediatric Hematology and Oncology
| | - Karoline Ehlert
- University Medicine Greifswald, Department of Pediatric Hematology and Oncology
| | - Winfried Barthlen
- Clinic of Pediatric Surgery, Ferdinand-Sauerbruch-Strasse 1Greifswald, Germany
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De Bernardi B, Di Cataldo A, Garaventa A, Massirio P, Viscardi E, Podda MG, Castellano A, D’Angelo P, Tirtei E, Melchionda F, Vetrella S, De Leonardis F, D’Ippolito C, Tondo A, Nonnis A, Erminio G, Gigliotti AR, Mazzocco K, Haupt R. Stage 4 s neuroblastoma: features, management and outcome of 268 cases from the Italian Neuroblastoma Registry. Ital J Pediatr 2019; 45:8. [PMID: 30634996 PMCID: PMC6329141 DOI: 10.1186/s13052-018-0599-1] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/14/2018] [Accepted: 12/19/2018] [Indexed: 01/03/2023] Open
Abstract
BACKGROUND Infants diagnosed with stage 4 s neuroblastoma commonly experience spontaneous disease regression, with few succumbing without response to therapy. We analyzed a large cohort of such infants enrolled in the Italian Neuroblastoma Registry to detect changes over time in presenting features, treatment and outcome. METHODS Of 3355 subjects aged 0-18 years with previously untreated neuroblastoma diagnosed between 1979 and 2013, a total of 280 infants (8.3%) had stage 4 s characteristics, 268 of whom were eligible for analyses. Three treatment eras were identified on the basis of based diagnostic and chemotherapy adopted. Group 1 patients received upfront chemotherapy; Group 2 and 3 patients underwent observation in the absence of life-threatening symptoms (LTS), except for Group 3 patients with amplified MYCN gene, who received more aggressive therapy. RESULTS The three groups were comparable, with few exceptions. Ten-year overall survival significantly increased from 76.9 to 89.7% and was worse for male gender, age 0-29 days and presence of selected LTS on diagnosis, elevated LDH, and abnormal biologic features. Infants who underwent primary resection ± chemotherapy did significantly better. On multivariate analysis, treatment eras and the association of hepatomegaly to dyspnea were independently associated with worse outcome. CONCLUSIONS Our data confirm that stage 4 s neuroblastoma is curable in nearly 90% of cases. Hepatomegaly associated to dyspnea was the most important independent risk factor. The cure rate could be further increased through timely identification of patients at risk who might benefit from surgical techniques, such as intra-arterial chemoembolization and/or liver transplantation, which must be carried out in institutions with specific expertise.
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Affiliation(s)
- Bruno De Bernardi
- Department of Hematology-Oncology, IRCCS Istituto Giannina Gaslini, Via Gaslini 5, 16147 Genoa, Italy
| | - Andrea Di Cataldo
- Department of Pediatric Hematology-Oncology, University Hospital, Catania, Italy
| | - Alberto Garaventa
- Department of Hematology-Oncology, IRCCS Istituto Giannina Gaslini, Via Gaslini 5, 16147 Genoa, Italy
| | - Paolo Massirio
- Department of Hematology-Oncology, IRCCS Istituto Giannina Gaslini, Via Gaslini 5, 16147 Genoa, Italy
| | | | | | - Aurora Castellano
- Department of Pediatric Oncology, Bambino Gesù Children’s Hospital, Rome, Italy
| | - Paolo D’Angelo
- Department of Pediatrics, University of Palermo, Palermo, Italy
| | - Elisa Tirtei
- Department of Pediatric Hematology-Oncology, Regina Margherita Hospital, Torino, Italy
| | - Fraia Melchionda
- Hematology-Oncology Unit, Sant’Orsola-Malpighi Policlinic, Bologna, Italy
| | - Simona Vetrella
- Department of Hematology-Oncology, Santobono-Pausilipon Children’s Hospital, Naples, Italy
| | | | | | - Annalisa Tondo
- Department of Hematology-Oncology, Anna Meyer Children’s Hospital, Florence, Italy
| | | | - Giovanni Erminio
- Epidemiology and Biostatistics Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Anna Rita Gigliotti
- Epidemiology and Biostatistics Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Katia Mazzocco
- Pathology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Riccardo Haupt
- Epidemiology and Biostatistics Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
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Klimovich GV, Ramos-Irizarry CT, Falk GA, Loux T. Development of abdominal compartment syndrome secondary to tumor lysis in an infant with disseminated stage 4 neuroblastoma despite decompressive laparotomy. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2016. [DOI: 10.1016/j.epsc.2015.11.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022] Open
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Lew T, Chauhan A, Vasquez R, Warrier R. Massive Hepatomegaly With Respiratory Distress in a Newborn. Clin Pediatr (Phila) 2015; 54:907-9. [PMID: 26137965 DOI: 10.1177/0009922815591095] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Affiliation(s)
- Tony Lew
- University of Queensland Clinical School at Ochsner, New Orleans, LA, USA
| | - Aman Chauhan
- LSUHSC/Children's Hospital, New Orleans, LA, USA
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Fernandez-Pineda I, Sandoval JA, Davidoff AM. Hepatic metastatic disease in pediatric and adolescent solid tumors. World J Hepatol 2015; 7:1807-1817. [PMID: 26207162 PMCID: PMC4506938 DOI: 10.4254/wjh.v7.i14.1807] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2015] [Revised: 04/21/2015] [Accepted: 05/28/2015] [Indexed: 02/06/2023] Open
Abstract
The management of hepatic metastatic disease from solid tumors in adults has been extensively described and resection of metastatic liver lesions from colorectal adenocarcinoma, renal adenocarcinoma, breast cancer, testicular cancer, and neuroendocrine tumors (NET) have demonstrated therapeutic benefits in select patients. However, there are few reports in the literature on the management of hepatic metastatic disease in the pediatric and adolescent populations and the effectiveness of hepatic metastasectomy. This may be due to the much lower incidence of pediatric malignancies and the higher chemosensitivity of childhood tumors which make hepatic metastasectomy less likely to be required. We review liver involvement with metastatic disease from the main pediatric solid tumors, including neuroblastoma and Wilms tumor focusing on the management and treatment options. We also review other solid malignant tumors which may have liver metastases including germ cell tumors, gastrointestinal stromal tumors, osteosarcoma, desmoplastic small round cell tumors and NET. However, these histological subtypes are so rare in the pediatric and adolescent populations that the exact incidence and best management of hepatic metastatic disease are unknown and can only be extrapolated from adult series.
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Weintraub M, Waldman E, Koplewitz B, Bloom AI, Gross E, Freeman AI, Revel-Vilk S. A sequential treatment algorithm for infants with stage 4s neuroblastoma and massive hepatomegaly. Pediatr Blood Cancer 2012; 59:182-4. [PMID: 22605456 DOI: 10.1002/pbc.23186] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2011] [Accepted: 04/08/2011] [Indexed: 01/06/2023]
Abstract
Infants with 4s neuroblastoma (NB) and massive hepatomegaly have a guarded prognosis and mortality approaches 30%. We report on eight patients with 4s NB and massive hepatomegaly treated with multiple modalities. One patient had spontaneous tumor regression. Three patients had progressive disease and responded to chemotherapy. Four patients progressed despite intravenous chemotherapy, of whom two died, and two were salvaged with hepatic intra-arterial chemoembolization. Treatment of infants with stage 4s NB with massive hepatomegaly should be individualized based on disease course. A sequential approach with observation, intravenous chemotherapy, and intra-arterial chemoembolization, may improve the outcome of these infants.
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Affiliation(s)
- Michael Weintraub
- Department of Pediatric Hematology-Oncology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
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Ejike JC, Mathur M. Abdominal decompression in children. Crit Care Res Pract 2012; 2012:180797. [PMID: 22482041 PMCID: PMC3318199 DOI: 10.1155/2012/180797] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2011] [Revised: 11/17/2011] [Accepted: 12/30/2011] [Indexed: 12/15/2022] Open
Abstract
Abdominal compartment syndrome (ACS) increases the risk for mortality in critically ill children. It occurs in association with a wide variety of medical and surgical diagnoses. Management of ACS involves recognizing the development of intra-abdominal hypertension (IAH) by intra-abdominal pressure (IAP) monitoring, treating the underlying cause, and preventing progression to ACS by lowering IAP. When ACS is already present, supporting dysfunctional organs and decreasing IAP to prevent new organ involvement become an additional focus of therapy. Medical management strategies to achieve these goals should be employed but when medical management fails, timely abdominal decompression is essential to reduce the risk of mortality. A literature review was performed to understand the role and outcomes of abdominal decompression among children with ACS. Abdominal decompression appears to have a positive effect on patient survival. However, prospective randomized studies are needed to fully understand the indications and impact of these therapies on survival in children.
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Affiliation(s)
- J. Chiaka Ejike
- Division of Pediatric Critical Care, Department of Pediatrics, School of Medicine, Loma Linda University, Loma Linda, CA 92354, USA
| | - Mudit Mathur
- Division of Pediatric Critical Care, Department of Pediatrics, School of Medicine, Loma Linda University, Loma Linda, CA 92354, USA
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Haberal M, Ozcay F, Sevmis S, Karakayali H, Moray G, Torgay A, Coskun M, Demirhan B, Sarialioglu F, Arslan G. Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy. Pediatr Transplant 2008; 12:358-62. [PMID: 18266801 DOI: 10.1111/j.1399-3046.2008.00887.x] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.
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Affiliation(s)
- Mehmet Haberal
- Department of General Surgery and Transplantation, Baskent University, Ankara, Turkey.
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Boztug K, Kiely E, Roebuck DJ, Gaze M, Begent J, Brock P, Michalski A. Successful treatment of MYCN amplified, progressive stage 4S neuroblastoma in a neonate with hepatic artery embolization in addition to multimodality treatment. Pediatr Blood Cancer 2006; 46:253-7. [PMID: 15926157 DOI: 10.1002/pbc.20407] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Stage 4S metastatic neuroblastoma (NB) has a favorable prognosis due to a high rate of spontaneous regression. Young infants risk lethal complications arising from hepatomegaly, which can develop rapidly despite treatment. MYCN oncogene amplification confers a significantly worse prognosis. We describe a 4-week-old neonate with MYCN-amplified stage 4S NB complicated by gross hepatomegaly causing rapidly progressive respiratory, hepatic, and renal failure. The child remains in remission 3 years after hepatic artery embolization, radiotherapy, standard, and high-dose chemotherapy. Embolization of the hepatic artery, with classical treatment, is feasible and safe at this age and may contribute substantially to the management of high-risk patients.
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Affiliation(s)
- Kaan Boztug
- Department of Oncology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, United Kingdom
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Abstract
BACKGROUND Stage 4S neuroblastoma is associated with a high rate of spontaneous maturation and involution, with survival rates of 70-90%. There is little long-term follow-up data describing the disease status or late effects. The aim of this study was to assess the clinical outcome and imaging findings in long-term survivors of 4S neuroblastoma. METHODS The patient population was identified from a single centre over 26 years. Twenty-five of 31 consecutive patients were long-term survivors. Five died from disease progression and one from cerebral palsy related complications. All survivors underwent clinical examination. Abdominal ultrasound scanning, liver function tests, hepatitis viral screen, and urinary catecholamines were performed. RESULTS The mean age at diagnosis was 8 +/- 9 weeks with a mean age when studied of 11 years and 10 months +/- 8 years. Twenty of 25 had no significant clinical findings, three had disease associated clinical abnormalities (neurological, multiple subcutaneous nodules). Three patients had treatment related effects (small testes, urethral stricture, radiation induced soft tissue hypoplasia, post-surgical Horners syndrome). Persistant adrenal enlargement and calcification was noted in three patients. Twelve patients had abnormal liver ultrasound findings ranging from mildly coarse echotexture to structural changes without evidence of hepatic dysfunction or infection. Treatment did not correlate with abnormal hepatic ultrasound findings. CONCLUSIONS The majority of long-term survivors of stage 4S neuroblastoma have no clinically or radiologically significant sequelae but do have residual abnormalities. These findings have implications for subsequent management of unrelated medical conditions in this patient group.
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Affiliation(s)
- G A Levitt
- Department of Haematology/Oncology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
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Abstract
Neuroblastoma is the most common extra-cranial solid tumor in children and has a heterogeneous clinical presentation and course. Clinical and biologic features of this disease have been used to develop risk-based therapy. Patients with low-risk disease can be treated with surgery alone. Patients with intermediate-risk features have an excellent prognosis after treatment with surgery and a relatively short course of standard dose chemotherapy. Unfortunately, most children with neuroblastoma present with advanced disease. More than 60% of patients with high-risk features will succumb to their disease despite intensive therapy including a myeloablative consolidation. Research efforts to understand the biologic basis of neuroblastoma and to identify new, more effective therapies are essential to improve the outcome for these children.
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Affiliation(s)
- Robert E Goldsby
- Division of Pediatric Hematology/Oncology, University of California, San Francisco 94143-0106, USA
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